SOP: antibody-associated autoimmune encephalitis
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(2020) 2:1
Neurological Research and Practice
STANDARD OPERATING PROCEDURE
Open Access
SOP: antibody-associated autoimmune encephalitis Rosa Rössling1,2 and Harald Prüss1,2*
Abstract Background: Antibody-mediated and paraneoplastic autoimmune encephalitides (AE) present with a broad spectrum of clinical symptoms. They often lead to progressing inflammatory changes of the central nervous system with subacute onset and can cause persistent brain damage. Thus, to promptly start the appropriate and AEspecific therapy, recognition of symptoms, initiation of relevant antibody diagnostics and confirmation of the clinical diagnosis are crucial, in particular as the diseases are relatively rare. Aim: This standard operating procedure (SOP) should draw attention to the clinical presentation of AE, support the diagnostic approach to patients with suspected AE and guide through the necessary steps including therapeutic decisions, tumour screening and exclusion of differential diagnoses. Method: Based on existing diagnostic algorithms, treatment recommendations and personal experiences, this SOP gives an overview of clinical presentation, diagnostic procedures and therapy in AE. Additional information is provided within an accompanying text and a table describing the most important autoantibodies and their characteristics. Results: The initial steps of the AE flow chart are based on clinical symptoms and the patient’s history. Assignment to paraneoplastic or antibody-mediated AE is sometimes clinically possible. Diagnostics should include MRI, EEG and CSF analysis with antibody panel diagnostic. Definite AE can be diagnosed if the underlying antibody is compatible with the clinical presentation. Classification of probable AE may be possible even with negative antineuronal autoantibodies if the clinical presentation and laboratory abnormalities are highly suggestive of AE. The confirmed AE diagnosis requires immediate initiation of immunotherapy. Conclusion: The SOP facilitates the recognition of patients with AE and presents the necessary diagnostic and therapeutic steps. Keywords: Autoimmune, Encephalitis, Limbic encephalitis, Antibody, Paraneoplastic, NMDAR
Introduction Autoimmune encephalitis (AE) is an often rapidly progressive inflammatory neurological disease with subacute onset. The best characterized and most common form of AE is anti-NMDA receptor (NMDAR) encephalitis, defined by cerebrospinal fluid (CSF) IgG antibodies targeting the NMDA type glutamate receptor. Patients can present with altered mental status, reduced levels of consciousness, deficits in working/short-term memory that * Correspondence: [email protected] 1 Department of Neurology and Experimental Neurology, Charité – Universitätsmedizin Berlin, CharitéCrossOver, R 4-334 ,Charitéplatz 1, 10117 Berlin, Germany 2 German Center for Neurodegenerative Diseases (DZNE) Berlin, Berlin, Germany
develop in usually less than 3 months (frequently within < 6 weeks), and may show psychosis or new epileptic seizures [1]. AE comprises antibody-mediated and
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